INTRODUCTION: Esophageal achalasia is a primary motility disorder. Although the exact pathogenesis is unknown, autoimmune, and neurodegenerative processes seem to be involved similarly to neurodegenerative and/or demyelinating disorders (NDDs). We hypothesized that the prevalence of NDD may be higher among patients with achalasia and vice versa as the background pathogenetic mechanisms are similar. METHODS: This was a prospective, comparative questionnaire-based study. Patients with achalasia and patients with NDD were enrolled. Selected patients with achalasia were thoroughly examined by a neurologist and selected patients with NDD were examined by a gastroenterologist to confirm or rule out NDD or achalasia. We assessed the prevalence of both achalasia and NDD and compared them with their prevalence in general population. RESULTS: A total of 150 patients with achalasia and 112 patients with NDD were enrolled. We observed an increased prevalence of NDD among patients with achalasia (6.0% (9/150); 95% CI (confidence interval): 3.1-11.2%) as compared to the estimated 2.0% prevalence in general population (p = 0.003). Although 32 out of 112 patients (28.6%) with NDD reported dysphagia, we did not observe significantly increased prevalence of achalasia in these patients (1.8% (2/112) vs 0.8% in general population, p = 0.226). CONCLUSION: The prevalence of NDD was significantly higher among patients with achalasia (6.0%) compared to general population (2.0%), suggesting an association of these disorders. Large-volume studies are necessary to confirm this finding.

• Publikační typ
• časopisecké články MeSH

Cíle: Zpracování souboru dětských pacientů operovaných pro cholesteatom za celistvým bubínkem. Určení incidence kongenitálního cholesteatomu ve sledovaném souboru. Metodika: Retrospektivní studie, která hodnotí 21 dětských pacientů operovaných v letech 2007 - 2017 na Klinice dětské otorinolaryngologie LF MU a FN Brno (KDORL) pro cholesteatom za celistvým bubínkem. Výsledky: V období 2007 až 2017 bylo provedeno na KDORL celkem 202 primárních operací pro cholesteatom. Ve 21 případech (10,4 %) se jednalo o cholesteatom za celistvým bubínkem, z toho v 11 případech (5,45 %) o kongenitální cholesteatom. Od roku 2013 je patrný stoupající trend záchytu cholesteatomu za celistvým bubínkem. Nejčastějším příznakem onemocnění byla převodní nedoslýchavost, z hlediska rozsahu cholesteatomu stadium III dle Potsica a nejčastěji zvoleným chirurgickým postupem byla atikoantrotomie. 17 pacientů podstoupilo operaci ve druhé době (second look), která byla ve 4 případech pozitivní (23,5 %). Závěr: Včasná diagnostika a léčba cholesteatomu za celistvým bubínkem u dětí zvyšuje šanci na kompletní odstranění cholesteatomu, snižuje riziko recidivy a zlepšuje vyhlídky na dobrý sluch. K jeho záchytu v nižších stadiích je nejdůležitější mikrootoskopie. V pokročilejším stadiu se může projevit zhoršením sluchu, proto by každá nevysvětlitelná jednostranná převodní nedoslýchavost měla být došetřena.

Objective: The processing of the group of children operated on for a cholesteatoma behind an intact tympanic membrane. The determination of the incidence of congenital cholesteatoma in the examined group. Methodology: This retrospective study evaluates 21 child patients from the Clinic of Pediatric Otorhinolaryngology LF MU and FN Brno (KDORL), who were operated in the period 2007 - 2017 on for cholesteatoma behind an intact tympanic membrane. Results: During a period 2007 – 2017, a total of 202 primary operations were performed for cholesteatoma. In 21 cases (10,4%) there was a cholesteatoma behind an intact tympanic membrane and in 11 cases of it there was the congenital cholesteatoma. From the year 2013 there is an increasing trend of the detection of cholesteatoma behind an intact tympanic membrane. The most common sign of this disease was the conversion deafness and taking into account the range of cholesteatoma (stadium III according to Potsic), the atticoantrotomy was the most frequent used surgical technique for a treatment. There were 17 patients indicated for a second operation (second look), which was positive in 4 cases (23,5%). Conclusion: The early diagnostics and the treatment of cholesteatoma behind an intact tympanic membrane in children increase the chance of complete removal of cholesteatoma, decrease the risk of recidivism as well and improve the prospects for a good hearing. The microotoscopy is the most important examination for the early detection of cholesteatoma. At an advanced stage there could be the hearing impairment and because of this reason every inexplicable unilateral hearing impairment should be always explore.

• Klíčová slova
• kongenitální cholesteatom,
• MeSH
• cholesteatom středního ucha * chirurgie   MeSH
• dítě MeSH
• lidé MeSH
• retrospektivní studie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH

Calcium is essential for proper muscular function and metabolism. Myopathy with high creatinkinase activity can be a rare manifestation of hypocalcemia of various origin, such as vitamin D deficiency, hypoparathyroidism, pseudohypoparathyroidism (PHP). 16-year old previously healthy boy was admitted to intensive care unit with convulsions lasting for three minutes and a transient loss of consciousness. Laboratory results revealed severe hypocalcemia (total S-Ca < 1.0 mmol/L; normal 2.2-2.6 mmol/L), hyperphosphatemia (S-P 2.8 mmol/L; normal 0.6-1.6 mmol/L). Serum creatinkinase (S-CK) activity was 32 μkat/L (normal 0.57-2.45 μkat/L). Other basic biochemical parameters including creatinine, troponin, alkaline phosphatase were within normal values. Calcemia was gradually corrected within two weeks by intravenously and orally administered calcium and by cholecalciferol. S-CK reached a maximum of 222 μkat/L on day 4 and dropped to 7.2 μkat/L on day 14. Boy had no myalgias, neither clinical signs of myopathy. Echocardiography was normal with normal myocardial contractility, without any signs of calcification. The serum level of parathyroid hormone (S-PTH) was high (12 pmol/L; normal 0.7-5.5 pmol/L), fully compatible with the diagnosis of PHP. Molecular analysis revealed pseudohypoparathyroidism type Ib (PHPIb).In conclusion, manifest tetany and even mild myopathy with very high S-CK can occur in hypocalcemic patients and usually resolves after normalization of hypocalcemia.

• MeSH
• hypokalcemie etiologie   MeSH
• kreatinkinasa krev   MeSH
• lidé MeSH
• mladiství MeSH
• pseudohypoparatyreóza diagnóza   MeSH
• záchvaty etiologie   MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Pea seed-borne mosaic virus (PSbMV) significantly reduces yields in a broad spectra of legumes. The eukaryotic translation initiation factor has been shown to confer resistance to this pathogen, thus implying that translation and proteome dynamics play a role in resistance. This study presents the results of a proteome-wide analysis of Pisum sativum L. response to PSbMV infection. LC-MS profiling of two contrasting pea cultivars, resistant (B99) and susceptible (Raman) to PSbMV infection, detected >2300 proteins, 116 of which responded to PSbMV ten and/or twenty days post-inoculation. These differentially abundant proteins are involved in number of processes that have previously been reported in the plant-pathogen response, including protein and amino acid metabolism, stress signaling, redox homeostasis, carbohydrate metabolism, and lipid metabolism. We complemented our proteome-wide analysis work with targeted analyses of free amino acids and selected small molecules, fatty acid profiling, and enzyme activity assays. Data from these additional experiments support our findings and validate the biological relevance of the observed proteome changes. We found surprising similarities in the resistant and susceptible cultivars, which implies that a seemingly unaffected plant, with no detectable levels of PSbMV, actively suppresses viral replication. BIOLOGICAL SIGNIFICANCE: Plant resistance to PSbMV is connected to translation initiation factors, yet the processes involved are still poorly understood at the proteome level. To the best of our knowledge, this is the first survey of the global proteomic response to PSbMV in plants. The combination of label-free LC-MS profiling and two contrasting cultivars (resistant and susceptible) provided highly sensitive snapshots of protein abundance in response to PSbMV infection. PSbMV is a member of the largest family of plant viruses and our results are in accordance with previously characterized potyvirus-responsive proteomes. Hence, the results of this study can further extend our knowledge about these pathogens. We also show that even though no viral replication is detected in the PSbMV-resistant cultivar B99, it is still significantly affected by PSbMV inoculation.

• MeSH
• chromatografie kapalinová MeSH
• eukaryotické iniciační faktory MeSH
• hrách setý virologie   MeSH
• interakce hostitele a patogenu * MeSH
• nemoci rostlin virologie   MeSH
• odolnost vůči nemocem genetika   MeSH
• Potyvirus patogenita   MeSH
• proteomika metody   MeSH
• rostlinné viry MeSH
• tandemová hmotnostní spektrometrie MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

• MeSH
• akutní nemoc klasifikace   MeSH
• aplikace orální MeSH
• dexamethason aplikace a dávkování   farmakokinetika   farmakologie   MeSH
• diferenciální diagnóza MeSH
• dítě MeSH
• epiglotitida * diagnóza   farmakoterapie   klasifikace   MeSH
• lidé MeSH
• management nemoci MeSH
• posouzení stavu pacienta MeSH
• prednisolon aplikace a dávkování   farmakokinetika   farmakologie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH

Phytohormones are orchestrators of plant growth and development. A lot of time and effort has been invested in attempting to comprehend their complex signaling pathways but despite success in elucidating some key components, molecular mechanisms in the transduction pathways are far from being resolved. The last decade has seen a boom in the analysis of phytohormone-responsive proteins. Abscisic acid, auxin, brassinosteroids, cytokinin, ethylene, gibberellins, nitric oxide, oxylipins, strigolactones, salicylic acid--all have been analyzed to various degrees. For this review, we collected data from proteome-wide analyses resulting in a list of over 2000 annotated proteins from Arabidopsis proteomics and nearly 500 manually filtered protein families merged from all the data available from different species. We present the currently accepted model of phytohormone signaling, highlight the contributions made by proteomic-based research and describe the key nodes in phytohormone signaling networks, as revealed by proteome analysis. These include ubiquitination and proteasome mediated degradation, calcium ion signaling, redox homeostasis, and phosphoproteome dynamics. Finally, we discuss potential pitfalls and future perspectives in the field. This article is part of a Special Issue entitled: Plant Proteomics--a bridge between fundamental processes and crop production, edited by Dr. Hans-Peter Mock.

• MeSH
• Arabidopsis metabolismus   MeSH
• biologické modely * MeSH
• proteiny huseníčku metabolismus   MeSH
• proteom metabolismus   MeSH
• proteomika metody   MeSH
• regulátory růstu rostlin metabolismus   MeSH
• signální transdukce fyziologie   MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH

Publikace se zaměřuje na výpovědi a příběhy českých zdravotnických klaunů. Určeno široké veřejnosti.

• MeSH
• dějiny 21. století MeSH
• terapie smíchem MeSH
• vtip a humor jako téma MeSH
• zdravotnický personál MeSH
• Check Tag
• dějiny 21. století MeSH
• Publikační typ
• osobní vyprávění MeSH
• Geografické názvy
• Česká republika MeSH

• Konspekt
• Fyzioterapie. Psychoterapie. Alternativní lékařství
• NLK Obory
• psychoterapie
• humanitní vědy a umění
• NLK Publikační typ
• příběhy

Porodní traumatismus je soubor projevů poškození plodu a novorozence účinkem mechanických sil v průběhu porodu. Může dojít k poranění CNS, vnější části neurokrania, kostí, měkkých tkání (nervů, svalů, kůže), smyslových a parenchymatózních orgánů. V našem případě došlo k masivnímu krvácení pod galea aponeurotica u fyziologicky probíhajícího porodu dítěte s následně diagnostikovanou vrozenou koagulopatií.

A birth trauma is a set of neonatal injuries due to mechanical powers which are created during delivery. Central nervous system, external parts of neurocranium, bones, tissues (nerves, muscles, skin), sensory and parenchymatous organs can be involved. In our case massive neonatal hemmorhage under galea aponeurotica developed during spontaneous vertex delivery. Subsequently a congenital coagulopathy was diagnosed in the newborn.

• Klíčová slova
• extrakraniální krvácení, krvácení pod galea aponeurotica,
• MeSH
• diseminovaná intravaskulární koagulace diagnóza   etiologie   terapie   MeSH
• faktor VIII * aplikace a dávkování   škodlivé účinky   terapeutické užití   MeSH
• genetické nemoci vrozené diagnóza   etiologie   terapie   MeSH
• hemofilie A * diagnóza   etiologie   terapie   MeSH
• hemoragický šok diagnóza   etiologie   terapie   MeSH
• klinické laboratorní techniky MeSH
• koagulopatie diagnóza   etiologie   MeSH
• lidé MeSH
• novorozenec MeSH
• poporodní krvácení diagnóza   prevence a kontrola   terapie   MeSH
• porodní poranění novorozence * komplikace   prevence a kontrola   terapie   MeSH
• statistika jako téma MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• Publikační typ
• kazuistiky MeSH

Posttranslational modifications (PTMs) of proteins represent fascinating extensions of the dynamic complexity of living cells' proteomes. The results of enzymatically catalyzed or spontaneous chemical reactions, PTMs form a fourth tier in the gene - transcript - protein cascade, and contribute not only to proteins' biological functions, but also to challenges in their analysis. There have been tremendous advances in proteomics during the last decade. Identification and mapping of PTMs in proteins have improved dramatically, mainly due to constant increases in the sensitivity, speed, accuracy and resolution of mass spectrometry (MS). However, it is also becoming increasingly evident that simple gel-free shotgun MS profiling is unlikely to suffice for comprehensive detection and characterization of proteins and/or protein modifications present in low amounts. Here, we review current approaches for enriching and separating posttranslationally modified proteins, and their MS-independent detection. First, we discuss general approaches for proteome separation, fractionation and enrichment. We then consider the commonest forms of PTMs (phosphorylation, glycosylation and glycation, lipidation, methylation, acetylation, deamidation, ubiquitination and various redox modifications), and the best available methods for detecting and purifying proteins carrying these PTMs. This article is part of a Special Issue entitled: Posttranslational Protein modifications in biology and Medicine.

• MeSH
• lidé MeSH
• posttranslační úpravy proteinů * MeSH
• proteom chemie   izolace a purifikace   metabolismus   MeSH
• proteomika metody   MeSH
• zvířata MeSH
• Check Tag
• lidé MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH

The conventional stoichiometry of the oxidation of elemental sulfur by ferric iron in Acidithiobacillus ferrooxidans was not in agreement with our experimental data in terms of ferrous iron and proton formation. Reaction modelling under the actual conditions of bacterial activity resulted in a different stoichiometry, where additional iron species participate in the process to affect the number of released protons. The suggested reaction equation may more accurately predict the intensity of environmental acidification during the anaerobic bioprocess.

• MeSH
• Acidithiobacillus metabolismus   MeSH
• anaerobióza MeSH
• energetický metabolismus MeSH
• oxidace-redukce MeSH
• síra metabolismus   MeSH
• železité sloučeniny metabolismus   MeSH
• železnaté sloučeniny metabolismus   MeSH
• železo metabolismus   MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH